This patient presented with bilateral hearing loss, dizziness, and skin lesions. Imaging showed bilateral vestibular schwannomas, trigeminal schwannomas, a cervical tumor, and pelvic mass. The patient was diagnosed with neurofibromatosis type 2 (NF2) based on the multiple tumors. NF2 causes bilateral vestibular schwannomas and other tumors and has a genetic basis. The patient underwent surgery to remove the right vestibular schwannoma and will receive gamma knife radiosurgery for the remaining tumors. Early diagnosis and treatment of NF2 is important to prevent complete deafness and other complications.

3. Patients with vestibular
schwannomas associated with
neurofibromatosis 2 represent a
special challenge because of the
risk of complete deafness.

4. Case details
A 23yrs male,presented to
ENT dept ,NMCH, with
• B/L impaired hearing since 4yrs
• Frequent falls since 4 years
• Ringing sensation in both ears since 4yrs
• Difficulty in walking 4 months

5. Clinical history
• 6 months back had one episode of tonic-clonic
seizures and controlled on anti-epileptic
medications
• Following seizures he had loss of
consciousness and post-ichtal headache
• Progressive weakness of all four limbs

6. Clinical history cont…
• No history of
– Vomiting
– Blurring of vision
– Diminution of vision
• No significant personal history

7. • He had a swelling over scalp and for the same
he was operated 2 years back
• All siblings and parents are healthy

8. Clinical examination
• GPE:
– Multiple café au lait macules over abdomen and
back (each 1-3 cm)
– Subcutaneous swellings over left parietal region and left sub mandibular region
- ? Neurofibromas
• PR – large, firm pelvic mass
• Spine - normal

9. – ENT examination – revealed
– RNNIES- B/L AC>BC
– WEBERS-NO LATERALISATION
– ABC- REDUCED
NEUROLOGICAL EXAMINATION
• V – corneal reflex absent b/l,

10. • Motor:
– Bulk – N
– Tone - spasticity in lower limbs
– Power:
• 3-4 in right sided limbs
• 4-5 in left sided limbs
• Sensory – normal
• Cerebellar signs:
– Axial and appendicular ataxia
• Gait – wide based, swaying to either side

11. • AUDIOMETRY
• PTA:
– Right ear loss 75 dB (SNHL)
– Left ear loss 60 dB
• SRT – 90 dB
• SDS – very poor (out of proportion to
PTA loss)
• BERA-absent waves
• ACOUSTIC REFLEX- absent
• OAE-absent
• VEMP-absent

12. •MRI brain T2 axial images
•showing multiple illdefined lobulated
extra axial hyperintense lesions in
bilateral CP angles .
•Extending from IAM & widening it.
•Obstructive hydrocephalous on Lt
side,dilatation of temporal ,occipital
horns of lateral sinus

13. • MRI post contrast
images show multiple
tumors in bilateral CP
angles.
• With homogenous
enhancement

14. • Saggital T2 MRI
cervical spine
showing illdefined
heterogenous an
intradural lesion at
C5-6 level

15. • Large well defined
T2 hyperintense
mass in presacral
region
,compressing
adjacent pelvic
organs.

16. • DIAGNOSIS:
– NEUROFIBROMATOSIS TYPE 2
• BILATERAL VESTIBULAR SCHWANNOMAS
• BILATERAL TRIGEMINAL SCHWANNOMAS
• CERVICAL SCHWANNOMA / EPENDYMOMA
• PELVIC ?SCHWANNOMA / NEUROFIBROMA

17. • TREATMENT:
– Right retrosigmoid
suboccipital craniectomy
and excision of right
vestibular schwannoma
•Further plan:
For left schwannoma, other neuromas – GKRS
Cervical cord tumor – follow-up
Pelvic mass - ?surgery

18. DISCUSSION
U/L VS
• sporadic ,95%
• 30-60yrs
• Not hereditary
• Both sexes involved equally
B/L VS
• a/w NF2 genetic disorder
with fault in chrom22.
• 5% incidence
• 50% chance of inheriting
from affected parent.
• Late adolescence/ early
childhood.

19. Management
• Surgery
• Radiation
• Supportive and rehabilitation
• Counselling and screening for family members

20. • Unlike the solitary sporadic tumours that tend
to displace the cochlear nerve, tumours
associated with NF2 tend to form nodular
clusters that engulf or even infiltrate the
cochlear nerve.
• Complete resection may not always be
possible. Radio surgery has been performed
for patients with NF2.

21. CONCLUSION
• Though B/L vestibular schwannoma is a rare
presentation ,if pt present with b/l progressive
SNHL, balancing disorder ,skin lesions suspect in
the view of NF2
• Rule out family history
• Early diagnosis &management is necessary to
prevent complete deafness & serious
consequences

22. • Post operatively the patient had no
deterioration in hearing revealed by PTA
• Pt was followed uP for 1 yr & he is doing well